Term
| Sickle Cell Disease due to mutation in ? ? of adult hemoglobin (Hb), the O2 transport protein in red blood cells |
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| Sickle cell disease only manifests when ? in common type, Glu6-to-Val mutation in Beta chain causes formation of hydrophobic patch on surface of beta chain |
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| One symptom of sickle cell disease is ?, as well as swelling of extremities, higher risk of stroke and infection |
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| A treatment for sickle cell disease is hydroxyurea, which causes increased expression of ? Hb chain |
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| What is the major RBC protein? |
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| Total oxygen content = O2 on Hb (SaO2) + ? ? |
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| What is the heme (prosthetic group-tightly bound organic molecule) + the globin chain called? |
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| Going from oxygenated to deoxygenated Hb, Iron does not change ? ? |
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| What is an O2 storage protein? |
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| What is an O2 carrier protein |
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| ? is found in the muscle,? is found in the erythrocyte |
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| What has 1 Heme group (protoporphyrin IX) |
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| What has 4 hemes (protoporphyrin IX) |
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| ? has 4 globin chains (tetramer) (2 alpha types + 2 beta types) |
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| what has 1 globin chain (monomeric) |
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| What has no allosteric effectors and a HYPERBOLIC curve |
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| What has allosteric effectors (H+, CO2, 2,3-BPG, Cl-) and a SIGMOIDAL CURVE |
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| ? = PO2 required for 50% saturation of the Hb (Ys = 0.5) A measure of oxygen affinity |
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| What is a measure of the degree of cooperative interaction between oxygen binding sites. Binding of first O2 facilitates binding of 2nd, etc. (sigmoidal, nH (+) ) |
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In the Hill Equation
Ys = (PO2)^nH / { (P50) ^ nH + ( PO2) ^ nH}
What does Ys represent??? |
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| What has an nH of 1.0 and a P50 of 2 to 3 torr and no cooperativity?!?! |
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| What has an nH of 2.8 and a P50 between 26 and 27 torr with positive cooperativity |
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| Hill number can't exceed ?, which is the maximum cooperativity number. |
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| At birth 70 - 90% HbF is asymptomatic for beta chain defect. Thus ? and ? ? ? are not evident. |
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Definition
| beta-thalassemia and sickle cell disease |
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Term
| ? : Diminished production of alpha or beta chains. Leads to unstable forms of Hb that are not fully functional (i.e., no cooperativity or allosterism) and have low solubility |
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| The allosteric protein of adult hemoglobin is inhibited by ?, ?, 2,3-BPG, and Cl-, which lower ? ? |
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| In hemoglobin, O2 binding changes position of ? ion |
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| Why does Hb release 66% of bound O2? Because of ? binding which lowers O2 affinity by stabilizing T-state. It promotes the release of oxygen and is an allosteric inhibitor that binds in center of T-state tetramer. |
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| Fetal hemoglobin (HbF) binds to ? less than adult Hb |
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| 2,3-BPG induces a shift to the ? of O2 dissociation curve |
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| ? Effect: Higher acidity (more H+) gives more oxygen delivery and pushes curve to the right. |
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| ? effect: CO2 lowers affinity of Hb for oxygen. Moves curve to the right. |
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| ? Effect: Cl- likewise promotes formation of T-state. Interacts with positive side chains of deoxy Hb and stabilizes ionic interactions. Moves curve to the right. |
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